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Cir Cir 2012;80:167-170

Unusual clinical presentation of astrocytoma in an adultJulio César Cortés-Medina* and Carlos Alberto Guerrero-Rascón**

* Departamento de Enseñanza Clínica e Investigación, **Neurocirugía, Hospital General Regional No. 1, Instituto Mexicano del Seguro Social, Morelia, Michoacán, México

Correspondence:Dr. Julio César Cortés MedinaHospital General Regional No. 1 IMSSDepartamento de Enseñanza Clínica e InvestigaciónAvenida Camelinas 1935, Col. Electricistas58290 Morelia, Michoacán, MéxicoTel: 01(443) 3122185E-mail: cortes.med@hotmail.com

Received for publication: 1-18-2011Accepted for publication: 6-8-2011

abstract

Background: Brainstem gliomas have a varied biological behavior based upon histopathological characteristics, local-ization and related diseases. The objective of this study is to present the clinical case of a grade II (diffuse) astrocytoma in an adult with an atypical clinical presentation including initial hiccup and dysphagia without evidence of clinical primary gastroenterology pathology.Clinical case: We describe the case of a 51-year-old male with a 4-year history of hiccup, oropharyngeal dysphagia and neurological clinical elements of tongue fasciculations, quadriparesis, generalized hyperreflexia due to astrocytoma with atypical localization in the floor of the fourth ventricle and histopathological diagnosis of low-grade diffuse glioma.Conclusions: Hiccup is a nonspecific symptom infrequently associated with neurological disorders. Adequate semiol-ogy of oropharyngeal dysphagia would help us to identify neurological entities such as in the case presented here. Additionally, with signs of upper and lower motor neuron we should suspect the possibility of central nervous system abnormalities of functional and organic etiology. Computed axial tomography and magnetic resonance imaging studies are important elements for diagnosis.

Key words: hiccup, oropharyngeal dysphagia, astrocytoma grade II, central nervous system

malignancy. These data are useful for establishing progno-sis in these types of tumors where, in pediatric patients, sur-vival is less than during adulthood.3-6 Adequate semiology of symptoms, coupled with a detailed physical examination and magnetic resonance imaging (MRI) are the best diag-nostic elements for these tumors. The aim of this study is to present the case of a grade II astrocytoma in an adult with atypical clinical presentation.

Case report

We present the case of a 51-year-old male who was admit-ted in June 2010 to the Regional General Hospital No. 1 in Morelia, Michoacan. His medical history is notable for be-ing a member of Jehovah's Witnesses, along with a 12-year history of chronic alcohol abuse. The patient’s illness be-gan 4 years prior with the presence of intermittent hiccups whose frequency progressed to once every 2 months (dura-tion ~2 days) and became continuous during the 7 months prior to admission. He also reported dysphagia to solids and liquids with a sensation of choking, nasal voice, dysphonia, and dysarthria, in addition to weight loss (not quantified). Directed questioning was negative for odynophagia, heart-burn or drooling.

Neurological examination showed tongue fasciculations and hypotrophy predominantly on the left side and no gag reflex. The remainder of the examination of the cranial

introduction

Hiccups and dysphagia are nonclinical data characteristic of a diffuse type of tumor such as astrocytoma because it has higher prevalence during the pediatric age.1,2 However, these symptoms are most common between the third and fourth decades of life. Additionally, for brain stem tumors, the characteristic symptoms are due to the compression of lower cranial nerves causing hemiparesis, ataxia, nausea and vomiting.

Diffuse astrocytoma is characterized as a slow-growing tumor capable of invading nearby structures. A close rela-tionship exists between age, histological type and grade of

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nerves was normal. Strength of the extremities was quanti-fied as 3/5 in upper extremities as well as lower extremities consistent with the Daniels scale7 and generalized hyper-reflexia. Bilateral negative Babinski was demonstrated and, based on signs and symptoms, dysphagia of central type neurological origin was suspected.

On endoscopic examination there was resistance to passing the cricopharyngeal cartilage without injury to the esophagus and chronic gastritis with mild atrophy in the antrum. Esophagogram reported an area of stenosis at the upper third of the esophagus and uncoordination at the cri-copharyngeal level. Also identified was decreased caliber of the distal esophagus and esophagogastric junction of a possible peptic origin. Laboratory values were normal.

Single phase cranial CT showed a hypodense rounded image at the margin of the fourth ventricle modifying its structure with calcification in its periphery (Figure 1). Brain MRI with gadolinium contrast showed a hypointense and cystic image appearance with lateralization to the left with growth in the fourth ventricle without causing hydrocepha-lus (Figure 2). MRI in sagittal cuts showed the presence of an extra-axial lesion with bulbar compression (Figure 3).

The patient underwent mid-suboccipital craniectomy with conventional technique of microsurgery, resection of

the lesion and dural plasty. Four weeks after surgery there was progressive improvement of neurological deficit, gait with left lateral propulsion and the base of support was ex-panded. Weight gain was shown after 2 months follow up. Histopathological results reported diffuse grade II astrocy-toma.

discussion

Hiccups and dysphagia, as in this case, are unusual symp-toms in patients with astrocytomas. The literature reports hiccups in 2% of cases, whereas dysphagia occurs in 15% of cases. Involvement of the hypoglossal nerve (cranial nerve XII) is present in 2%.8-10 The association of hiccups, dysphagia, and cranial nerve XII in the absence of esopha-geal, chest or neck diseases should point to an injury of the central nervous system. This may explain the involvement of the vagus nerve (cranial nerve X) and sensory and mo-tor impairment of the oropharyngeal phases of swallowing,

figure 1. Single-phase computed tomography. Rounded hypodense lesion in the margin of the fourth ventricle, lateralized to the left, mo-difying its structure with a calcification in its periphery.

figure 2. MRI: Coronal T1-weighted images with contrast (gadoli-nium): hypointense lesion in the posterior fossa with cystic appea-rance.

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taking into account differentiation from other syndromes such as lateral medullary syndrome, neuromuscular disease, generalized elevation of intracranial pressure, amyotrophic lateral sclerosis, oculopharyngeal muscular dystrophy, Parkinson's disease and metabolic encephalopathies such as uremia.11 Brain stem astrocytomas are presented in the region comprised from the cerebral aqueduct to the fourth ventricle and can be classified depending on their location as diffuse intrinsic pontine, tectal, and cervicomedullary. These tumors represent 2% of intracranial tumors in adults and 9.4% in children. The most common subgroup is repre-sented by low-grade diffuse astrocytoma.

Given the low frequency of stem astrocytomas in adults, a longer survival rate has been identified in this age group than vs. diffuse tumor in pediatric ages.12 Stem astrocyto-mas are less aggressive in adults than in children

Approximately 75% of the patients are <20 years, un-like the patient in this case who was in the fifth decade of life. Patient survival differs according to tumor location be-ing 80% at 5 years in mesencephalic tumors and <20% for those located in the pons and medulla. In diffuse intrinsic tumors of the stem, survival is <1 year.13 In this study the patient had a lesion under the fourth ventricle with bulbar compression which, based on its histology, in 27% of cases tends to show an anasplastic change.14 Histopathologically it can be classified, according to WHO, in degrees 1 to 4.15,16

Computed tomography (CT) usually shows a hypodense lesion with partial enhancement that rarely calcifies or

bleeds, with certain limitations due to the presence of mul-tiple artifacts due to bony elements of the posterior fossa. For these reasons we recommend MRI studies where brain stem gliomas show ring enhancement and edema associated with high-grade lesions.17,18 The patient had calcification in the periphery of the lesion, whereas MRI showed an in-fratentorial lesion without edema.

An important tool in the histopathological diagnosis is immunohistochemistry that allows the demonstration of tumor-associated antigens. This facilitates their clas-sification and provides a direct method for assessing the malignant potential and proliferative cellular capacity.19,20

The clinical, imaging and pathological experience has al-lowed establishment of prognostic factors.21 Favorable factors are considered to be focal, cystic-type lesions or those with exophytic component in the dorsum of the stem and whose presentation is in adulthood in association with neurofibromatosis type 1. Poor prognostic factors are le-sions with diffuse infiltration of the bridge, high mitosis and compromise of various cranial nerves.22 In accordance with the characteristics our patient demonstrated, progno-sis was favorable.

Neurofibromatosis type I (NF1) and brain stem tumors are diseases whose clinical features are headache, cranial neuropathies and symptomatic hydrocephalus associat-ed with variable course astrocytoma. Its frequency in the population with NF1 is unknown. However, Molloy23 et al. estimate in their follow-up that 4% presented the previous clinical entity in the population with NF1, suggesting close clinical observation and monitoring by imaging studies in order to assess disease progression.24

As a rule, all intrinsic brain stem tumors should be biop-sied to determine diagnosis and define treatment. Epstein and McCleary25 report that this procedure is not innocuous and that the pathology sample may not be representative. The tumors can be heterogeneous and it is believed that most are malignant. In the presence of these types of le-sions, surgical treatment is preferred and favorable results have been obtained in the exophytic type within the fourth ventricle and the cystic lesion with mural nodule. In this manner, a definitive diagnosis is achieved, resolving the dif-ferential diagnostic difficulties with other expansive lesions in this region26 as happened in this case.

Conventional radiotherapy offers a transitory improve-ment27,28 and chemotherapy is not effective in controlling primary or recurrent brain stem gliomas.29 Lack of effec-tiveness of chemotherapy in these types of tumors is justi-fied by their different biological behavior, with greater rates of resistance and lower rate of local drug penetration due to local vascular characteristics.30,31

Treatment options improve quality of life but do not im-prove survival.

figure 3. MRI, sagittal cut. Extra-axial lesion ECHO 1 (T1) of a cystic component causing bulbar compression.

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In conclusion, this case illustrates the importance of ade-quate semiology of hiccups and dysphagia in a patient with neurological clinical data that should lead physicians to suspect the possibility of CNS abnormalities. Physical ex-amination should be directed, comprehensive and specific to topographical areas responsible for functions that govern speech and swallowing, and only in this manner early di-agnosis and appropriate timely treatment will be achieved.

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